Physical Therapy for Hypermobility Spectrum Disorder
2/23/18
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PT for Hypermobility Spectrum Disorders
A Zebra Among Us: Recognition & Management of
Hypermobility Spectrum Disorders Combined Sections Meeting 2018
New Orleans, LA, February 21-24, 2018 LeslieNRussek,PT,DPT,PhD,OCS
ClarksonUniversity,Canton-PotsdamHospital,Potsdam,NYStephanieSaboPT,MPT
Cincinna:Children’sHospitalMedicalCenter,Cincinna:,OHJaneSimmondsDProf,MCSP,MACP,SFHEA
GreatOrmondIns:tuteofChildHealth,UniversityCollegeLondon,London,UKHeatherPurdin,MS,PT,CMPT
GoodHealthPhysicalTherapy&Wellness,Portland,OR
1
Disclosures The speakers have no financial or other conflicts of interest.
PT for Hypermobility Spectrum Disorders 2
Affiliations [email protected] [email protected] [email protected] [email protected]
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Learning Objectives Attheendofthiscourse,parDcipantswillbeableto:
1. Describetypicalclinicalpresenta:onofpa:entswithhypermobilityspectrumdisorder(HSD)throughthelifespan,includingpediatric,adolescent,andadultpa:entswithinthecontextoftheICFmodel.
2. Applythe2017Interna:onalClassifica:ontoiden:fyHypermobilitySpectrumDisordersandhEDS.
3. Proposeevidence-basedapproachesforphysicaltherapymanagementforindividualswithhypermobilityspectrumdisorders.
4. Recognizecommonchallengesandpi]allsworkingwiththesecomplexpa:ents.
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Outline • 25min:Introduc:ontoEDS,hEDS,HSD(LRussek)• 25min:Pediatriccase(StephanieSabo,presentedbyL.Russek)
– Exam,evalua:on,motordelays,motorcontrolissues,bracing• 25min:Adolescentcase(JaneSimmonds)
– Exam,evalua:on,stra:fiedmanagement,POTS,fa:gue,GIproblems,psychosocialissues
• 25min:Adultcase(HeatherPurdin)– Exam,evalua:on,chronicpain,MCAD,
• 20min:QA(panel)
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Many Types of EDS • Hypermobile (III): Loose joints, joint pain. Most common. • Classical (I & II): Velvety, stretchy, fragile skin. Common. • Vascular (IV): Possible arterial/organ rupture. Most serious. • Kyphoscoliosis: Joint laxity, muscle hypotonia,
developmental delay. Severe functional loss over time. • Arthrochalasia (VII): Congenital hip dislocation, lax joints. • Dermatosparaxis (VII): Severe skin fragility & bruising.
• Malfait, 2017
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Physical Therapy for Hypermobility Spectrum Disorder
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Vascular Ehlers-Danlos Syndrome
PT for Hypermobility Spectrum Disorders www.ehlersdanlosnetwork.org/vascular.html
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Classical Ehlers-Danlos Syndrome
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Many Names of Hypermobility • Generalizedjointhypermobility/laxity(GJH)
– Mightormightnothavepainorsymptoms• Jointhypermobilitysyndrome(JHS)&hypermobilitysyndrome
(HMS):termsodenusedbyrheumatologists• Ehlers-DanlosSyndrome–hypermobilitytype(EDS–HTortypeIII)
terminologyusedbygene:cists• NewlyproposedterminologyhypermobileEhlers-DanlosSyndrome
(hEDS)andHypermobilitySpectrumDisorder(HSD)• Wewillrefertoitas‘hypermobility’or‘HSD’
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Hypermobility Spectrum Disorder
Individual
JointLaxity
GJH
EDS
hEDS
Asymptomatic Mild Symptoms Severe Symptoms
GJH = (asymptomatic) joint hypermobility; EDS = Ehlers-Danlos Syndrome; hEDS = hypermobile EDS Castori, 2017
Localized-HSD Generalized-HSD Peripheral-HSD Historical-HSD
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Prevalence of EDS • HSDisthemostcommonsystemicinheritedconnec:ve:ssue
disorderinhumans(Tinkle,2017)– intheUKprevalenceofHSDassociatedwithchronicwidespreadpain
orseverelydisablingpain(Mulvey,2013)isalmostashighasfibromyalgia(Fayaz,2016)and100:meshigherthanrheumatoidarthri:s.(Humphreys,2013)
• Affects~10millionpeopleintheU.S.(Tinkle,2017)– 30xnumberofTHA/yr;60xnumberofACLreconstruc:ons/yr
• HSDinmusculoskeletalhealthcare:30-55%
• InOmaniwomen,probablynotthishighintheUS.(Clark,2011)• 80-90%ofallEDSishEDS(Tinkle,2017)
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Pathophysiology • Autosomaldominantconnec:ve:ssuedisorder• Thereisnogene:cmarkerknownforhEDS
– IncontrasttomostoftheotherformsofEDS• Heterogeneouspathophysiology
– TenascinXabnormality?– TypeIIIcollagenabnormality?– Exactconnec:ve:ssueabnormalityisunknown– (Tinkle,2017;Malfait,2017)
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Physical Therapy for Hypermobility Spectrum Disorder
2/23/18
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Common Signs & Symptoms • Musculoskeletal: joint hypermobility, subluxations/dislocations, sprains,
muscle spasm, TMD, flat feet, finger deformities, arthralgia, myalgia, fractures, pain, proprioceptive deficits, kinesiophobia
• Integumentary: stretchy skin, easy bruising, atrophic scarring, poor wound healing, frequent hernias
• Cardiovascular: dysautonomia, postural orthostatic tachycardia syndrome Other: gastritis, IBS, incontinence, developmental delay, poor coordination, anxiety, mast cell activation, organ prolapse
• Disability due to pain, fatigue, anxiety, depression (Castori, 2011; Colombi, 2015; Scheper, 2016; Tinkle, 2017)
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Major Comorbidities • Chronic pain: fibromyalgia, myofascial pain, OA, TMD
– Hyperalgesia (Scheper, 2017)
• Developmental delay in children • Dysautonomia: POTS, thermoregulation, gut, sexual
dysfunction • Mast Cell Activation Disorder: systemic inflammation • Gastrointestinal disorders: GERD, IBS, malabsorption
syndrome (Tinkle, 2017)
• Tethered cord syndrome (Henderson, 2017)
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HSD Through the Life Span 1. Hypermobile phase
– Hypermobile joints – Clumsiness/motor delay – Constipation/diarrhea – Abdominal hernias
2. Pain phase – Chronic musculoskeletal pain – Strains, sprains, dislocations – Unrefreshing sleep – Chronic fatigue – Memory/cognitive problems – Gastric reflux, abdominal pain – Paresthesias – Tachycardia – Incontinence/UTI
3. Stiffness phase – Widespread pain – Fatigue – Tendinosis/tendon rupture – Chronic gastritis – Stiffness
– (Castori, 2011; Tinkle, 2017)
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Evolving Diagnostic Criteria • Un:l2017:
– BeightonScale(Beighton,1973)mostodenusedforGJH• Carter-Wilkinson(Carter,1964)• Rotés/HospitaldelMar(Bulbena,1992)
– VillafrancheClassificaDonusedmostlybygene:cistsforchildren:EDS-HT(Beighton,1998)
– BrightonCriteriausedmostlybyrheumatologistsforadults:JHS(Grahame,1998)
• NewcriteriaforhEDS:“The 2017 international classification of the Ehlers-Danlos syndromes.”(Malfait,2017)
• NoclearguidelinesregardingHSD;Mustexcludeotherpoten:aldiagnoses
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2017 hEDS Diagnostic Criteria Mustmeetall3criteria:
1. Generalizedjointhypermobility
2. Featuresofheritableconnec:ve:ssuedisorder• Mustmeet≥2of3categories,A-C
3. Absenceofexclusioncriteria• (Malfait,2017)
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BeightonScore≥5/9
• 2:Bend5thfingerback>90°• 2:Touchthumbtoforearm• 2:Elbowhyperextension>10°• 2:Kneehyperextension>10°• 1:Palmstofloor,kneesstraight
• Prepubescent≥6/9• Pubescent-50years≥5/9• Over50years≥4/9
Pts with limited ROM for cause, add 1 point if ≥2/5 on the 5-Item Questionnaire: 1. Can you now (or could you ever) place your hands flat on the floor without
bending your knees? 2. Can you now (or could you ever) bend your thumb to touch your forearm? 3. As a child, did you amuse your friends by contorting your body into strange
shapes or could you do the splits? 4. As a child or teenager, did your shoulder or kneecap dislocate on more than
one occasion? 5. Do you consider yourself “double-jointed”?
(Malfait, 2017; diagram from Juul-Kristensen)
1: Generalized Joint Hypermobility
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2: Features of Heritable Connective Tissue Disorder
• Musthave≥2ofthefollowing3categories:A. Systemicmanifesta:ons
• ≥5of12op:onsB. FamilyhistoryC. Musculoskeletalcomplica:ons
• ≥1of3op:ons
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2A: Systemic Manifestations i. Unusuallysod/velvetyskinii. Mildskinhyperextensibility(forearm)iii. Unexplainedstraiae/stretchmarksiv. Bilateralpapulesofheelv. Recurrent/mul:pleabdominalherniasvi. Atrophicscarringin≥2sitesvii. Pelvicfloor,rectal,uterineprolapseviii. Dentalcrowdingorhigh,narrowpalateix. ArachnodactylybilateralSteinbergorWalkersignx. Armspan/height≥1.05xi. Mitralvalveprolapsemildorgreaterxii. Aor:crootdila:onMeetsSystemicManifestaDonsifYESto≥5items
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2A: Systemic Manifestations i. Unusuallysodorvelvetyskinii. Mildskinhyperextensibility(>1.5cmonvolar,non-
dominantforearm)iii. Unexplainedstraiae/stretchmarksinany�or
prepubertal�w/osignificantweightchange
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2A: Systemic Manifestations iv. Bilateralpiezogenicpapulesofheel*v. Recurrentormul:pleabdominalhernias(umbilical,
inguinal,crural;nothiatalhernia)*Piezogenic papules are sub-cutaneous fat herniations through the fascia; they may appear as white nodules only with weight bearing Malfait, 2017
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vi. Atrophicscarringinvolvingatleast2sites(notlikeclassicalEDS)
Malfait, 2017 Hypermobile EDS, Castori, 2015
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Hypermobile EDS Classical EDS 2A: Systemic Manifestations 2A: Systemic Manifestations
vii. Pelvicfloor,rectal,and/oruterineprolapseinchildren,�,nulliparous�w/omorbidobesity
viii. Dentalcrowdingandhighornarrowpalateix. Bilateralarachnodactyly
– Bilateral Steinberg OR bilateral Walker sign
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Physical Therapy for Hypermobility Spectrum Disorder
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2A: Systemic Manifestations x. Armspan/height*≥1.05xi. Mitralvalveprolapsemildorgreaterxii. Aor:crootdila:on,z-score>+2
*Arm span is from tip of middle finger to tip of middle finger
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2B: Family History • 1stdegreerela:veindependentlymeetsdiagnos:ccriteriaforhEDS
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2C: Musculoskeletal Complications 1. Pain≥2limbs,recurringdailyforatleast3months2. Chronicwidespreadpainfor≥3months3. Recurrentjointdisloca:onsorfrankjointinstabilityin
absenceoftrauma(aorb)a. 3+atrauma:cdisloca:onsofsamejointOR2+disloca:onsof2
differentjointsatdifferent:mesb. Medicalconfirma:onofjointinstabilityat2+jointsnotrelatedto
trauma
• Ifyesto≥1item(andnotduetootherconnecDveDssuedisorder,e.g.
lupus,RA),thenposiDveformusculoskeletalcomplicaDons
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2: Features of Heritable Connective Tissue Disorder
• Insummary,tomeetthecriterion:FeaturesofheritableconnecDveDssuedisorder
• Musthave≥2ofthefollowing3categories:
A. Systemicmanifesta:onsB. FamilyhistoryC. Musculoskeletalcomplica:ons(notexplainedbyanother
connec:ve:ssuedisorder)
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3: Absence of Exclusion Criteria TomeetthisCriterion,all3ofthefollowingmustbeABSENT:
1. Unusualskinfragility(shouldpromptconsidera:onofothertypesofEDS)
2. Otherheritableoracquiredconnec:ve:ssuedisorder(e.g,lupusorRA)
3. Neuromusculardisordersthatmaycausejointhypermobilitybymeansofhypotoniaorconnec:ve:ssuelaxity(e.g.,Marfan,otherEDS,OI,etc.)
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Patient Examination • Useabiopsychosocialapproach
• Lookforcontribu:ngfactorsaswellassigns,symptoms,&involved:ssues
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Physical Therapy for Hypermobility Spectrum Disorder
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International Classification of Functioning Disability and Health
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Engelbert , 2017; Pacey, 2014 PhD thesis
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Gastrointestinal
Dysautonomia
Fatigue
Psychological
Pain
Urogenital
Cardiovascular Neuromusculoskeletal
Symptom Profile Ninis, 2015
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Where To Start? Focus on patient’s primary activity & participation restrictions Engelbert, 2017
• Startatthebiggestcomplaint&worktowardsmallerissues• Lookforthekeystructures/problemsthatarecausingthe
pa:ent’sdeficits
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Principles of the Subjective Exam • Thorough,biopsychosocialinterview• Qualityoflifeassessment• Psychosocialassessmenttoolsasindicated(e.g.,depression,anxiety,etc.)
• Iden:fyhabitsandlifestylechoicesthatcontributetoprimarycomplaints– E.g.,sleephygiene,postures,ac:vi:es
• Whathashelped/harmedinthepast– Avoidiatrogenicinjuries(Bovet,2012)
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Outcome Measures • BristolImpactofHypermobility– Theonlycondi:on-specific– 55ques:ons– ~10mintocomplete– Validated– Reliability,MCIDnotyetdetermined
– (Palmer,2017)
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Physical Therapy for Hypermobility Spectrum Disorder
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What is Different in HSD? • TissuesinHSDaremorefragile(Tinkle,2017)• Maybedamagedbymoresubtlestressesorcontribu:ngfactors
(“theprincessandthepea”)– Areslowertoheal(Tinkle,2017)
• Gravitycanhaveabigimpact• Abnormalpainprocessing/hyperalgesia(Chopra,2017;Scheper,2017)• Mul:plecomorbidi:escancompoundproblems
– E.g.,Poorcoordina:on�microtrauma– Fa:gueorpain�decondi:oning– POTS:anxiety�muscletriggerpoints– MCAD�excessiveinflammatoryresponse
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Principles of the Physical Exam • Iden:fy:ssuescausingsymptoms• Iden:fystressorsaffec:ngthose:ssues
1. Isthereanimbalancebetweenlaxjointsand:ghtmuscles?2. Doespoorposture,alignmentorgravitystressjoints/muscles?3. Arebodymechanicsstretchingorstressingjoints/muscles?4. Ispoorpropriocep:onormotorcontrolleadingtoinstability?5. Whatiscausingmuscletriggerpoints?
• POTStest,ifappropriate
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Interventions • PaDenteducaDon!!!• Exercise(appropriate)• Painmanagement,focusingonself-care• Assis:veandortho:cdevices?• Manualtherapy?
(Engelbert,2017;ClinicalGuidelines;Chopra,2017)
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Patient Education EducateandempowerthepaDent/family
• Paineduca:on&self-management• Bodymechanics/ergonomics
• Ortho:cs,braces,&splintsif/whenneeded• Appropriateexercise/ac:vity• Sleephygiene&fa:guemanagement• POTSself-management• Psychological&socialwellness,relaxa:onstrategies• Dietandfluidmanagement• Otherissues:GIdysfunc:on,MCAD,incon:nence,etc.Refertootherprofessionalsasappropriate(Engelbert,2017;Chopra,2017)
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Exercise Prescription • Propriocep:on,stabiliza:on,motorcontrol&coordina:on
• Strengthening• Appropriatestretching,stabilizingasneeded• Cardiovascularcondi:oning
– “GradedExerciseTherapy”• (POTS-specificexercise)(Engelbert,2017;Palmer,2014)
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Pain Management (in Clinic) • Physiologicalquie:ng• Biofeedback• TENS(trialforhomedevice)• Manualtherapy• Dryneedling(iflegalinyourstate)• Shi<topa=entself-management
– Avoidextensiveuseofmodali=esintheclinic(Chopra,2017;Engelbert,2017)
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Medications • Li|ledefini:veresearchevidenceformedica:ons• NSAIDsfortrueinflamma:on
– NSAIDsmayslow:ssuehealing,aggravateGIsymptoms• Tricyclics,an:-seizure,SNRImedsforneuropathicpain• Topicalanalgesicsandan:-inflammatorymedica:ons• Acetaminophen• Cau:ons:
– Opiatesforshorttermuseonly– Musclerelaxersmayaggravateinstability
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Chopra, 2017 Tinkle, 2017
Barriers To Treatment • Nega:vepastexperienceswithPT(Bovet,2016)• Iatrogenicinjuries(Bovet,2016)• Barrierstodoingexercise:(Simmonds,2017)
– Fear– Pain– Fa:gue
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Applying Evidence-Based Practice
• Althoughmoreresearchisavailablenow,manyques:onsaboutop:maltreatmentremainunanswered
• Wethereforerelyontheprinciplesofevidence-basedprac:ce
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Review • Hypermobility-relatedcomplaintsarecommon
– Hypermobilityaffectsmostbodysystems• The2017diagnos:ccriteriaforhEDSarerestric:ve
– ConsiderHypermobilitySpectrumDisorders• PTexamina:onshouldbebiopsychosocial(ICFmodel)
– Accountfor:ssuefragility– Lookforcontribu:ngfactors
• PTmanagementshouldaddresscontribu:ngfactorsPT for Hypermobility Spectrum Disorders 46
Case Examples • Theremainderofthissessionwillbethroughcaseexamples– Pediatriccase:StephanieSabo,presentedbyL.Russek
• Addressesassessment,motordelays,motorcontrolissues,bracing– Adolescentcase:JaneSimmonds
• Addressesassessment,stra:fiedmanagement,POTS,fa:gue,GIproblems,psychosocialissues
– Adultcase:HeatherPurdin• Addressesassessment,chronicpain,MCAD
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Managing Pediatric Patients with HSD
Case and slides provided by Stephanie Sabo, PT, MPT
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Physical Therapy for Hypermobility Spectrum Disorder
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Common Presentation in Children
• Difficultywithprolongedwalkingorstanding• Legpainworseineveningoratnightandwith/aderac:vity• Legpainsymptomsrelievedbyrubbingorheat• Historyof“growingpains”• Difficultysi~nginchairatschool,figi:ng• Trips,falls,beinglabeled“clumsy”(Bernie,2011;Adib,2005)• Grossmotordelays(Tirosh1991,Davidovitch1994,Bernie2011)• Coordina:ondifficul:es(Kirby2007)
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Developmental Condition vs. Hypermobility in Young Children?
• Standardizedmotorcompetencytestsarenotadequate– Donotcapturethefullimpactofjointhypermobilityonmotorfunc:onandqualityoflife
– Qualityofmovementandcompensa:onsshouldbethefocusoftheassessment
– Childrencanodenachieveindividualmotoritems,butnotefficientlyorrepe::vely
– (Remvig,2011)PT for Hypermobility Spectrum Disorders 50
Prevalence
• 34-35%inschoolagechildren• (Arroyo,1988;Remvig,2011;Junge,2013)
• NOgenderdifferencefoundat10y/o
• (Remvig,2011)
• Postpubertalgenderdifferences• (Quatman,2008)
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Additional Hx: Prior Therapies • Itisimportanttoaskaboutwhetherthechildhashadpriortherapyand
whetheriswasperceivedashelpful– Some:mespriortherapyreportedtobeofli|lebenefitandworsenedsymptoms– Askifhypermobilitybeingtargetedinpriortherapies;ifhypermobilitywasnot
recognized,lesslikelythattherapywassuccessful• Authorssuggestthefollowingpoten:alreasonsforpreviouslyfailed
therapies:– Techniquesbeingtooaggressive– Dura:ontoshort– Frequencytohigh– Failingtoappreciatefullscopeofinterven:onsrequired
(Keer, 2003; Hakim 2003)
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Referral Sources • Pediatrics• Rheumatology• Gene:cs• DevelopmentalPediatrics• Orthopedics• PainManagement
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Referral Sources • TheEDS/hypermobilitypopula:ondoesn’tfitanyspecialtyarea
perfectly• Jointhypermobilityand/oritsimpactonfunc:onmaybeiden:fied
duringtreatmentofanothercondi:on,soitishelpfultoscreen• Children:
– Mayormaynotcomewiththisdiagnosisonthereferral– Mayhaveseenmanyphysiciansorservicesmul:ple:meslookingfor
answers• Pa:entandfamilyodenexpressfrustra:onswithdelayindiagnosis
orlackofexplana:onforsymptoms.
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• MotherwithhEDS• Over2-hourdrivefromtherapy• Parentsseparated• Limitedinvolvementofbiologicalfather
IMPAIRMENTS PARTICIPATION
PERSONAL FACTORS ENVIRONMENT
• Paininankles&knees,7/10-10/10– Worsewithac:vity&atnight
• Headaches• Poorposture• Poorbalance• Abnormalgait• Jointhypermobility• Muscle:ghtness:HS,heelcords• Poormotorcontrol&motorskills
• Difficultywalking>20minutes• Difficultysi~nginchairatschool
• Troubleatschool• Troublewithsocialfunc:on,playingwithfriends
Tommy,age4
• Anxious• A|en:ondeficit/hyperac:vitydisorder
• Self-imageas“clumsy”anduncoordinated
I want to play just like all the
other kids
ACTIVITY
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Tommy: Initial Presentation • 4yearoldmalereferredtoPTbyPediatricRheumatologist
– Onsetofsymptoms~1yearago,withincreasedcomplaintsinthelast6months
– Motherwithbehavioranda|en:onconcerns:“doesn’teversits:ll”
– SenttoRheumatologybyprimarycarephysician– 6monthslaterseenbyGene:cs(perRheumatologyReferral)
• Motherwithhypermobility/EDS
Insert subject
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Tommy: Initial Presentation • Paininanklesandknees2-3x/week
– 7/10onNumericRa:ngScale(NRS)– increaseswithphysicalac:vity– increasesinevening/night
• Upto10/10onNRS;motherhastakenhimtoemergencydepartmentforpain
• Bilateralhipandkneexrays-normal• Usesrubbingandmedica:onforrelief(e.g.,overthecounter
TylenolorMotrin)• Pa:entalsohascomplaintsofheadaches
Insert subject
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Tommy: Findings • Posture:
– Shouldersforward/rounded,increasedlordosis/anteriorpelvic:lt,genurecurvatum,calcanealvalgusandpesplanus,genuvalgum,externalrota:onatfeet
• Gait:– Decreasedkneeflexion,overprona:onofrearfoot-midfoot,audiblefootslap,
ERatfeet• ROM:
– HipER85o,HipIR70o,KneeExtension+8o,AnkleDorsiflexion30o,Ankleinversion60o
• Flexibility:– Hamstrings-40o;heelcords20o
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Measuring Flexibility • Assessmuscleflexibility
– Differen:atemuscleflexibilityandjointmobility
• Isolatemusclelengthvs.jointlaxity– Reinforceneedtostrengthenmusclesaroundloosejoints
• Muscularimbalances– Commonlyseelimita:onsinhamstringsandgastrocnemiusmusclegroups
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Tommy: Findings • Singlelimbstance:right:4-5seconds;led:4-5seconds
– Notedunsteadiness,withlateraltrunklean,trunkflexion,increasedanklestrategies,andadduc:onofoppositeLE
• Bridgeinhooklyingfor3secondsx3reps– Notedunsteadinessanddecreasedeccentriccontroluponlowering
• Miniwallsqua~ngfor10secondsx3reps– Noteddifficultywithmiddlerangecontrol:pushingkneeintogenu
recurvatumandpelvisforwardintoAPTwithoutcueing• Modifiedheelraises:singleUEsupportfor3secondsx3reps
– Notedtoini:allycompletethroughfullankleplantarflexionbutlockingoutankles;whencompletedinmiddlerange,demonstratedunsteadinessandsteppingstrategies
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Measuring Strength • Assesscorestrength
– Qualityofmovementisveryimportant:focusonac:va:onofkeymusclegroupsthroughhips,coreandpelvis• Forexample:bridgingmayini:atethroughtrunkextensionandlackcoreac:va:on
– Under-u:liza:onofkeyposturalmusclesiscommon-Transverseabdominus,mul:fidus,gluteals,serratusanterior
– Co-contrac:onisodenlacking/decreased– (Falkerslev,2013;Jensen,2013;Grahame,2009)
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Common Strength Deficits
• Pts have difficulty maintaining stable, neutral postures and effectively controlling movement
• Efficient use of proper positions to accomplish tasks is not natural
• Pts often initially demonstrate a full crab walk position when asked to perform a bridge
• Even athletic patients can have difficulty sustaining a neutral core with simple bridging
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Strength
• Assessextremitystrengthusingfunc:onalstrengthtes:ng(nomanualmuscletes:ng):– Alltes:ngshouldbedoneinneutraljointposi:ons,where
strengthandenduranceareodendecreased– Avoidposi:onsatendofjointrange
• lockedjointsareassociatedwithligamentousdependency– Assessthequalityofmuscleco-contrac:on– Lookformuscleimbalances
• Oden,keyposturalmusclesareveryweakandnotproperlyu:lized
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Tommy: Ankle Strength
• With heel raises, Tommy positions ankles in end range to help stabilize
• He is unable to maintain a static position
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Tommy: Leg Strength Wall sits: • Note wide base of support with genu
valgum and increased pronation/ calcaneal valgum and knee flexion
• He has muscle endurance limitations and genu recurvatum with knee extension
• He has overall poor quality of movement and poor mid range control
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Balance and Proprioception • AssessbalanceandpropriocepDon
– Observecompensatorypa|ernsofmovement– Assessforalteredawarenessofbodyposi:oninspace(common)
• Mul:plestudiesconfirmpropriocep:vedeficits:– Lowerlimbpropriocep:onisdecreasedinBenignJointHypermobilitySyndrome
– Decreasedpropriocep:oninUEjoints(fingers)(Smith,2013;Rombaut,2010;Fatoye,2009;Sahin2008;Schubert-Hjalmarsson,2012)
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Balance and Proprioception
Single leg stand: • Observe duration of
single limb stance AND the quality of the movement
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Balance and Proprioception
Devia:onstolookfor• Lateral trunk lean (gluteus medius weakness) • Unsteadiness • Trendelenburg (hip drop) • Trunk flexion • Adduction of opposite leg using other limb for stability • Increased ankle strategies • Pronation through midfoot • Locking out knee • Internal rotation of stance limb
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Tommy: Intervention/Treatment Plan • FrequencyforPTservices:recommendedfollowupvisit
~4-6weeksaderevalua:on;however,pa:entwaslosttofollowupfor6monthsduetofamilyhavingsocialconcerns
• EducaDon:jointprotec:on,ac:vitymodifica:on,sodjoints• OrthoDcs:fi|edwithortho:csatevalua:on• HEP:bridging,resistedhipabduc:on,squats,passive
hamstringstretch• Treatments:completedHEP4x/weekfromevalua:onun:l
6monthfollowupvisit
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Basic Principles of Intervention • Beginlowlevelforbaseline
– Odenneedtobeginingravityeliminatedposi:onsand/oruseisometrics
• Progressatslowrate• Considerthewholeperson
– Notjustonejointata:me– Nonmusculoskeletalmanifesta:ons
• Customizetreatmentbasedonindividualneedsandgoals (Celletti, 2013)
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Joint Protection • Posturalre-educa:onandawareness
– Reducesstressonmusclesandjoints– Reducepainandfa:gueover:me
• Focusonawarenessofneutraljointposi:ons.Avoid:– Kneeandelbowhyperextension– Anteriorpelvic:lt/hiphanging(forwardand
laterally)– Wsi~ng– Roundedshoulderandforwardhead (Rombaut, 2012)
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Joint Protection
• Pa:entsshouldbeinstructedhowtoavoidoverstretchingjointsandtoreducemechanicalstressonjoints– Forexample:individualscanprac:cefindingandmaintaininga“sodknee”
posi:on,withkneesneutralorveryslightlyflexed
• Lackofposturalmuscleac:va:onreinforcesposturaldeficits• Avoidexcessivejointmovement,whichcouldleadtotears
andruptureofthesod:ssuessurroundingthejoint• (Rombaut,2012)
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Controlled Stretching • Paya%en(ontotechnique:
– Stabilizesurroundingjointstostretchspecific:ghtmuscleswithoutstressingotherstructures
• Contrastpa:ent’sini:alposi:onforstandinghamstringstretch(top)withperformanceAFTERinstruc:oninproperform(bo|om)
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Orthoses: Summary of Evidence • Thereislimited,inconclusiveevidence,evenasitrelatestothegeneral
“flatfeet”diagnosisinthepediatricpopula:on• Systema:creviewsfindthatstudiesusedifferentpa:entpopula:onsand
widelyvaryingtypeoforthoses (Evans,2011)• Noinforma:ontoconsistentlysupportusingortho:csornot• Planusfootinearlylifeisassociatedwithadultdegenera:vejointdisease
andinterven:onisindicated (Gross,2011)• Anecdotalexperiencewithuseofortho:csinthehypermobilepopula:on
isveryposi:ve (LocalCCHconsensus,2016)
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Minimal Control Orthotics
• Providesomesupporttopronated(flat)feet
• Canreducestressonalllowerextremityjoints
• Customizable
Cascade DAFO Fast Fit http://cascadedafo.com
Vasyli orthotics www.vasylimedical.com
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Models of Therapy • ConsultaDve:Emphasisisonhomeexerciseprogramsthatareperiodicallyupdatedbythetherapist
• Intermediate:1-3x/wk• Intensive:Frequentsessionsoverashortperiodof:me
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Consultative Model • Emphasisonhomeprogrammingandselfmanagement(odenusedinini:alstages)– Homeexerciseprogramestablishedatini:alsession– Educa:onwithchildandcaregivers:posture,jointprotec:on…– Ortho:cs,ifindicated
• Followupplanestablished– Individualized,emphasizingselfmanagement– Frequencyoftherapyisdeterminedattheevalua:on– Follow-upoden4to6weekslater
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Intensive Treatment Model • Mul:disciplinaryprogram,including
OT,PT,MD,Psychology,ChildLife– Seekstoprovideknowledge,aswellas
aphysicalboost– Providestoolsforselfmanagement– ProvidessocialInterac:ons– Par:cipantsbecometheirownexperts– Facilitateshomeadherenceand
lifelongmanagement– IncreasesindependencewithHEP (Bathen, 2013; Birt, 2013)
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Example Intensive Model • 2-weekprogram• Par:cipantsgroupedbygenderandage• AllwithEDSand/orjointhypermobilitycondi:ons• Benefits:
– IntensiveexposuretoPTempowersparentsandchildren– Par:cipantsgainknowledge,skillsandconfidencetoadaptexercisesandself-
managesymptoms
• Adolescentsfacedthemostdifficul:eswithfinding:me,privacyandmo:va:ontodotheexerciseprogramathome
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Intensive Model (2 week program) • Programstarted2011
• Outcomemeasurespreandpost:– PedsQL,COPM,TampaScaleKinesiophobia
• Psychologyusescogni:vebehavioraltherapyandgroupsessions
• MusicincorporatedinexercisesessionsandwithChildLife
• ChildLifehelpspa:entswritele|ersofencouragementtothenextgroupcomingintotheprogram
• HEPbooksareconstructedwithpicturesofthepa:entdoingtheexercises
• (Celle~,2013;Castori,2012)
PT
MD
Child Life
Aqua-tics PSY
OT
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Outcome Measures • CanadianOccupa:onalPerformanceMeasure(COPM)
• PediatricQualityofLifeMeasure(PedsQL)• TampaScaleforKinesiophobia(TSK)
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Tommy: Outcome At 6 months: • Leg pain decreased from
daily to 1-2x/month • Improved quality of
movements • Improved ability to sustain
hold counts • Decreased postural
compensations
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Summary of Pediatric Case • Hypermobilepa:entsareinmostpediatriccaseloads• Func:onalimpactofjointhypermobilitypainandfa:gueissignificant
andisodenunder-appreciated• Standardtherapyfrequentlyfailsthesepa:ents• Specific,targetedjointstabiliza:on,neuromusculartrainingand
educa:onareneeded• Lowintensityandslowprogressionofinterven:onisindicated• Livescanbechanged!
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Thanks To: Stephanie Sabo PT, MPT
Physical Therapist II [email protected]
• Cincinnati Children's Hospital Medical Center Joint Hypermobility Team 2014. “Identification and Management of Pediatric Joint Hypermobility” CCHMC EBDM Website Guideline 43 pages 1-22.
Available at https://www.cincinnatichildrens.org
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Managing Adolescents with HSD and HEDS Jane Simmonds DProf, MCSP, MACP, SFHEA
Outline of Adolescent Case
• Consider issues which arise in adolescence
• Stratified approach to management Hypermobility Spectrum Disorders and Hypermobile Ehlers Danlos Syndrome
• Case study
• Plans for research
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Reflect on the key physiological and psychological changes during adolescence 1. Hormonal changes
2. Growth – Peak Height Velocity
3. Developing independence Seminal study (Kirk,1967) described three quarters of patients developed symptoms prior to age 15 years Hypermobile adolescents 2 times more likely to develop musculoskeletal problems than non hypermobile counterpart. Risk increases 12 fold if overweight (Tobias, 2013)
PT for Hypermobility Spectrum Disorders 88
DS
Joint Laxity
hEDS
Asymptomatic Mild Signs Significant Signs & Symptoms & Symptoms
GJH = (asymptomatic) joint hypermobility; hEDS = hypermobile EDS EDS = Ehlers-Danlos Syndrome
GJH
(Castori, 2017)
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SPECTRUM
SIMPLE/ACUTE INTERMEDIATE COMPLEX/CHRONIC
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STRATIFIED MANAGEMENT
SIMPLE/ EARLY
Episode of acute musculoskeletal injury, sprains, dislocation, subluxations
enthesopathies
(Pacey 2010; Smith 2005)
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STRATIFIED MANAGEMENT
SIMPLE/ EARLY
Episode of acute musculoskeletal injury, sprains, dislocation, subluxations
enthesopathies
(Pacey, 2010; Smith, 2005)
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STRATIFIED MANAGEMENT
SIMPLE/ EARLY
Episode of acute musculoskeletal injury, sprains, dislocation, subluxations
enthesopathies
SIMPLE/ EARLY Ice, electrotherapy, tape, support, exercise,
screen, education – rehabilitate and prevent
PT for Hypermobility Spectrum Disorders 93 PT for Hypermobility Spectrum Disorders 94
STRATIFIED MANAGEMENT
SIMPLE/ EARLY
Episode of acute musculoskeletal injury, sprains, dislocation, subluxations
enthesopathies
SIMPLE/ EARLY Ice, electrotherapy, tape, support, exercise,
screen, education – rehabilitate and prevent
INTERMEDIATE Recurrent episodes, series of episodes at
different sites, deconditioning, some central/ peripheral sensitization, mild
systemic conditions
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STRATIFIED MANAGEMENT
SIMPLE/ EARLY
Episode of acute musculoskeletal injury, sprains, dislocation, subluxations
enthesopathies
SIMPLE/ EARLY Ice, electrotherapy, tape, support, exercise,
screen, education – rehabilitate and prevent
INTERMEDIATE Recurrent episodes, series of episodes at
different sites, deconditioning, some central/ peripheral sensitization, mild
systemic conditions
INTERMEDIATE Physiotherapy modalities have temporary
effect, no effect or exacerbate Modified / adapted approach
Functional Restoration
(Engelbert 2017; Scheper 2017)
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Subjective Assessment
Listen and develop the therapeutic
alliance
Listen to the history carefully……. Explore expectations with young person and parent Identify problems – prioritize – Pain – local/ general/ acute/ chronic (sensitization) – Joint instability – subluxations, dislocations, clicking – Fatigue – sleep, fluid, diet – Anxiety - Low mood/ depression – Gastrointestinal dysmotility – Dysautonomia – Postural Tachycardia Syndrome (POTS) – Gynaecology and bladder problems – Allergies
Explore impact – Physical activity/ Sport/ Hobbies Physical Education – Social, School, General health – Family history and thorough developmental history **
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Subjective Assessment
Identify barriers to
rehabilitation
Listen to the history carefully……. Explore expectations with young person and parent Identify problems – prioritize – Pain – local/ general/ acute/ chronic (sensitization) – Joint instability – subluxations, dislocations, clicking – Fatigue – sleep, fluid, diet – Anxiety - Low mood/ depression – Gastrointestinal dysmotility – Dysautonomia – Postural Tachycardia Syndrome (POTS) – Gynaecology and bladder problems – Allergies
Explore impact – Physical activity/ Sport/ Hobbies Physical Education – Social, School, General health – Family history and thorough developmental history **
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Subjective Assessment
Identify personal strengths and
interests to drive rehabilitation
Listen to the history carefully……. Explore expectations with young person and parent Identify problems – prioritize – Pain – local/ general/ acute/ chronic (sensitization) – Joint instability – subluxations, dislocations, clicking – Fatigue – sleep, fluid, diet – Anxiety - Low mood/ depression – Gastrointestinal dysmotility – Dysautonomia – Postural Tachycardia Syndrome (POTS) – Gynaecology and bladder problems – Allergies
Explore impact – Physical activity/ Sport/ Hobbies Physical Education – Social, School, General health – Family history and thorough developmental history **
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Objective Assessment Careful active and passive joint range and muscle length Functional assessment * Posture and gait – compensatory patterns Sit to stand/ squat – gluteal, quadriceps Single leg dip Heel raise – tibialis posterior Balance – Single leg / Y Balance Test / Hop/ Jump Repositioning tests – proprioception/ kinaesthetic Strength/ activation (careful testing* - through range) Test for POTS (standing test…refer on)
Observe Carefully
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Dysautonomia/ Postural Tachycardia Syndrome (POTS)
Near syncope on standing Venous pooling with colour changes Tachycardia –standing and changing position Can result in massive anxiety Excessive heart rate on exercise Hyperventilation Heat intolerance Nausea Mathias (2011); Kizilbash (2014)
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Dysautonomia/ Postural Tachycardia Syndrome (POTS)
Near syncope on standing Venous pooling with colour changes Tachycardia –standing and changing position Can result in massive anxiety Excessive heart rate on exercise Hyperventilation Heat intolerance Nausea
Mechanisms Illness
Hormonal Deconditioning Hypermobility Mathias (2011); Kizilbash (2014)
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Chair
Walking
Chair
Artefact corrected heart rate
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Autonomic Testing Typical response POTS patient
Tilt Test Rise of ≥30 BPM adults Rise of ≥ 40 BPM in adolescents
Time Time Mathias (2011); Raj (2013); Kizilbash (2014)
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Autonomic Testing Typical response POTS patient
Time 10 Minute Standing Test – Alternative to Tilt Test
Time
Tilt Test Rise of ≥30 BPM adults Rise of ≥ 40 BPM in adolescents
Mathias (2011); Raj (2013); Kizilbash (2014)
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IMPAIRMENTS ACTIVITY PARTICIPATION
PERSONAL FACTORS ENVIRONMENT
Meet Helen 15 years
• Abletowalkfor15-20mins• Strugglingwithwri:ng• Strugglingtocarryschoolbag• Strugglingonpublictransport–duetosyncope
• Missingschool• Struggleswithdancing,notswimmingorplayingnetball
• Reducedsocialac:vitywithfriends(mainlyonline)
• Motherwithfibromyalgia• Youngerbrother–hEDS• Lovelyfriends
• Female• Highachiever–Astudent• Lowconfidence
• Widespreadhypermobility++• Recurrentshoulder,kneefingersubluxa:ons
• Persistentpainandfa:gue• Decondi:oned+• Anxiousandlowmood• Presyncopeandfastheartratewhenstanding(POTS)
• Dysmenorrhea• Bloa:ngandearlysa:ety
PMHx: Hypermobility detected in early life. Enjoyed being active. Recurrent injuries, subluxations. Physiotherapists treated single areas. PC: Never got on top of problem, injury after injury….now not coping ….
I want to do my exams…play sport,
dance …..go shopping
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Gastrointestinal
Dysautonomia
Fatigue
Psychological
Pain
Urogenital
Cardiovascular
Neuromusculoskeletal
Symptom Profile Ninis (2015)
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IMPAIRMENTS ACTIVITY PARTICIPATION
PERSONAL FACTORS ENVIRONMENT
Meet Helen 15 years
• Pa:entSpecificFunc:onalScale(PSFS)
• GoalA|ainmentScale
• PedsQL• (Physicalfunc:on,social,emo:onalandschool)
• SelfEfficacyScale(VAS)• CopingScale(VAS)
• PainVisualAnalogueScale(VAS)
• PedsQLMul:dimensionalFa:gueScale
• SingleLegDip• ModifiedStarExcursionBalanceTest(YBalance)
• TiltTest/StandTest• PedsQLGastroIntes:nalSymptomsandWorryScales
PMHx: Hypermobility detected in early life. Enjoyed being active. Recurrent injuries, subluxations. Physiotherapists treated single areas. PC: Never got on top of problem, injury after injury….now not coping ….
Relevant Outcome Measures
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Management
• Prioritize problems • Motivate and empower – partnership • Educate young person and family on all aspects of the condition • Agree goals (short and longer term) • Pain and fatigue management – including sleep routine • Movement correction • Exercise - recondition
(Engelbert, 2017)
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(Simmonds,2017)
Surveyof946paDentsUK
ExperiencesofPhysiotherapy
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Liaise with school teachers Dance teacher Coaches
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Dislocation and
Subluxation
Panic = muscle spasm Position the joint Analgesic Heat - Breath – Relax - Distract Give it time Do your usual thing… Ice, analgesia Support for a few days…carry on
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Limited evidence: wrist/hand neoprene splint not effective for hand pain or writing speed (small sample) (Frohlich, 2011)
Expertopinion-Judicioususe**Canbeveryhelpfulforwhenreturningfrominjuryandforfunc:on
Splinting and Tape
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Orthotics and Footwear
Cochrane Review Recommends supportive footwear and orthotics for flexible flat feet (Evans, 2011)
Improved gait efficiency in young people with Developmental Coordination Disorder and Hypermobility Syndrome (Morrison, 2013)
Expert opinion - Judicious use of orthotics or supportive footwear/ heal cup/ high tops
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Management of POTS • Advice – fluids, electrolytes/salt, compression tights
– Positioning, anti syncope manoeuvres • Medications prescribed (Midodrine, Fludrocortizone, Beta blockade)
• Respiratory physiotherapy – hyperventilation • Anxiety management – psychological support • Small meals, low carbohydrate diet and FODMAP • Graded cardiovascular exercise and resistance training – focus on lower limbs
– Morning exercise • Incorporating exercise to manage joint instability • Recumbent to upright exercise
(Mathias, 2011; Fu, 2011; George, 2013; Kizilbash, 2014)
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PREMISE FOR EXERCISE: Long term benefits of improved physical fitness and lower limb strength counteract orthostatic intolerance"• Increase blood volume"• Increase cardiac output"• Enhance vascular compression due to increased muscle mass and tone"• Improve endothelilial function"• Improve baro reflex function "
"
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PREMISE FOR EXERCISE: Long term benefits of improved physical fitness and lower limb strength counteract orthostatic intolerance"• Increase blood volume"• Increase cardiac output"• Enhance vascular compression due to increased muscle mass and tone"• Improve endothelilial function"• Improve baro reflex function "
"During exercise, people with POTS have a low stroke volume response to exercise – leads to light headedness, dizziness, dyspnoea and weakness!
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Application of Exercise • Individualise treatment based on needs and goals • Begin at a low level for baseline
– Weak and poor proprioception (Ferrell 2004; Engelbert 2017)
– Fear and pain (Simmonds, 2017)
• Progress slowly – exercise through range (Pacey 2013) • Considermotorcontrol(Roussel,2009)andengagethought(Boudreau2010)• Consider the whole person – kinetic chain
– Not just one joint at a time – Non musculoskeletal manifestations
• Hands on to teach (Simmonds, 2017)
• Make it relevant and fun and include the family (Birt, 2015)
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For Helen
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Pilates ….. Dance
(Simmonds, 2017)
For Helen
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For Helen
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PT for Hypermobility Spectrum Disorders 123 PT for Hypermobility Spectrum Disorders 124
STRATIFIED MANAGEMENT
SIMPLE/ EARLY Episode of acute musculoskeletal injury,
sprains, dislocation, subluxations enthesopathies
SIMPLE/ EARLY Ice, electrotherapy, tape, support, exercise,
screen, education – rehabilitate and prevent
INTERMEDIATE Recurrent episodes, series of episodes at
different sites, deconditioning, some central/ peripheral sensitization, mild
systemic conditions
INTERMEDIATE Physiotherapy modalities have temporary
effect, no effect or exacerbate Modified / adapted approach
Functional Restoration
COMPLEX LONG TERM Chronic, longstanding, severe, unremitting
pain with profound deconditioning/ comorbidities, disability
PT for Hypermobility Spectrum Disorders 125
STRATIFIED MANAGEMENT
SIMPLE/ EARLY Episode of acute musculoskeletal injury,
sprains, dislocation, subluxations enthesopathies
SIMPLE/ EARLY Ice, electrotherapy, tape, support, exercise,
screen, education – rehabilitate and prevent
INTERMEDIATE Recurrent episodes, series of episodes at
different sites, deconditioning, some central/ peripheral sensitization, mild
systemic conditions
INTERMEDIATE Physiotherapy modalities have temporary
effect, no effect or exacerbate Modified / adapted approach
Functional Restoration
COMPLEX LONG TERM Chronic, longstanding, severe, unremitting
pain with profound deconditioning/ comorbidities, disability
COMPLEX/ LONG TERM Multi disciplinary management programme using functional andcognitive behavioural
approaches (Bathen 2014)
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Summary for Functional Rehabilitation Time to listen reassurance, educate, empower (young person and family)
Condition specific education, advice and support
Give HOPE, agree GOALS, and be CREATIVE
Treat the treatable – prioritise
Pain and fatigue management (young person and parent)
Improve movement – use your hands
Strengthen weak muscle groups
Improve cardiovascular fitness
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Future Research and Education
• Understand the factors which influence development of symptoms • Validate and agree outcome measures • Understand what works best for young people • Intervention trials • Education patients and professionals
PT for Hypermobility Spectrum Disorders 128
Acknowledgments Patients and families Professor Rodney Grahame Rosemary Keer Dr Nelly Ninis Dr Hanadi Kazkaz Dr Alan Hakim Professor Christopher Mathias Colleagues in the Hypermobility Unit
PT for Hypermobility Spectrum Disorders 129
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Adults with HSD
ByHeatherPurdin,MS,PT,[email protected]
Guido Daniele http://www.guidodaniele.com
PARTICIPATION
PERSONAL FACTORS
ENVIRONMENT
• Widespreadhypermobility• AllogradHSandAchillesusedtostabilizeRshoulderanditsdisloca:ngagain
• PastHxlumbardiscectomy• Weightgain40#sincestoppingpainmedslastyear
• UterineProlapse• Dizzinessandtachycardia• TMJandribsubluxa:ons• Widespreadhives&swelling
• Strugglingtocareforfamilyandhome
• ⬇︎Churchinvolvement
• Female• Driven• Frustratedbymedicalcare• KidswithhEDSneedsupport
• Suppor:vehusband• Sickkids/neardeathexperience
• Recentlyrelocated• StresslevelhighduetonewChurchassignment
I need to look after my
family
ACTIVITY • Limitedarmfunc:onduetoshoulderinstability
• Limitedmobility• PainlimitsADLandIADL
BODY STRUCTURE/FUNCTION
132
MeetMindy,37y/ohomemaker
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Gravity affects EDS/HSD Pre-operaDveshoulder
subluxaDonNormalshoulderx-ray
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Gravity is a problem InferioranteriorsubluxaDon Deltoid,uppertrap,rotatorcuffset
PT for Hypermobility Spectrum Disorders 134
Subluxed Shoulder Causes Neck/Jaw Malalignment
Downsloped R shoulder pulls lower neck to R and head corrects to L
Set shoulder brings head back to neutral
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Types of Pain • Iden:fythetype(s)ofpain
– Nocicep:ve:mechanical,inflammatory– Neuropathic:peripheral,(central)– Centralsensi:za:on:neuroplas:city
• Differentpainistreateddifferently
136 PT for Hypermobility Spectrum Disorders
Pain Source
Quality Medications/Referral
Exercise Other Intervention
Mechanical Localized, occurs with certain motions. Sensitized by inflammation
NSAIDs? Primary MD
Strength/Mobility Balance throughout kinetic chain Proprioception
Bracing, taping, Ergonomics training, Muscle setting
Inflammatory Burning in broad area not dermatomal, bruisy
NSAIDs, Allergy meds, MCAD Primary MD, Allergy
Pool Proprioceptive input Mindful movement
Modalities, Tool assisted scraping, Dietary guidance
Nerve Burning in a peripheral nerve or dermatomal pattern, searing
Gabapentin, Lyrica, SNRI’s, LDN, Triptylines Primary MD, Neuro, Nutrition?
Nerve flossing Mindful movement Cardio External focus
Posture training to address entrapped nerves, positions of slack
Central Sensitization
Whole body pain, difficult to localize, wind-up, allodynia
SNRI’s, triptylines, LDN Primary MD, Psych
Cardio Mindful movement External focus
Meditation Breathing Biofeedback 137
PT for Hypermobility Spectrum Disorders
(Chopra, 2017; Castori, 2012)
What is MCAD?
PT for Hypermobility Spectrum Disorders 138 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf.
Slide from Anne Maitland’s presentation at 2017 EDS Global Learning Conference
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MCAD Presentation
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Slide from A. Maiteland, EDS Global Learning Conference, 2017 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf.
Mindy’s Symptoms - Hives,redrash- Brainfog- Diges:veissues- Bladderirritability- Swellinginlimbs,supraclavicular
- Elevatedurinehistamines
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PT for Hypermobility Spectrum Disorders 141 Slide from A. Maiteland, EDS Global Learning Conference, 2017 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf.
Manage Mast Cell Activation • Iden:fytriggers:
– Alcohol,heat,medica:ons(NSAIDs,an:bio:cs,narco:cs),allergens– Foodsensi:vi:es– Excessiveexercise
• Managephysicalandemo:onalstress• Exerciseregularly,inspiteoffa:gue• Advocateforpa:entge~ngonMastCellStabilizersandmeds
thatblockchemicalmediatorslikean:-histamines– sendpa:enttoMDwithresearchar:cles
– (Akin, 2010; Moulderings, 2011; Seniviratne, 2017; Theoharides 2015)
142 PT for Hypermobility Spectrum Disorders
Dietary Advice • 37%ofpeoplewithIBShavehEDS• FODMAPdietmaybehelpful(expertopinion)
– Fructose,Oligosaccharides,Disaccharides,Monoamines,andPolyols– BacteriathriveonFODMAPfoods/dysbiosiscausesMCAD– MonashUniversity,MelborneAustrialia(Fikree,2017)
• Otherrecommenda:onsarespecula:ve– HeidiCollins,MDdiet–avoidchemicals,gluten,takesupplementstoimprove
nutri:on,reducehistamines– Lowhistaminediet(especiallywithMCAD)– Avoidhardfoodsandexcessivejawmovements(ice,gums,etc.)toavoidTMD– Avoidbladderirritantfoods(e.g.,coffee,citrusproducts)– Avoidlargemeals(especiallyofrefinedcarbohydrates)
143 PT for Hypermobility Spectrum Disorders
Sources of Pain • Don’tjustlookforsymptoma:c:ssue–findthecauseof:ssuesymptoms– E.g.:UTTrPmaybecausingHA,butpoorpropriocep=onandDNFmotorcontrolcauseUTTrP
• Chronicpain-consider– Psychosocialfactors– Stress– Childhoodtrauma
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HSD Through the Life Span 1. Hypermobile phase
– Hypermobile joints – Clumsiness/motor delay – Constipation/diarrhea – Abdominal hernias
2. Pain phase – Chronic musculoskeletal pain – Strains, sprains, dislocations – Unrefreshing sleep – Chronic fatigue – Memory/cognitive problems – Gastric reflux, abdominal pain – Paresthesias – Tachycardia – Incontinence/UTI
3. Stiffness phase – Widespread pain – Fatigue – Tendinosis/tendon rupture – Chronic gastritis – Stiffness
– (Castori, 2011; Tinkle, 2017)
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Adverse Childhood Experience • Specificadversechildhoodexperiences(ACEs)increaseriskofchronicpain– Verbalorsexualabuse,parentalpsychopathy,Earlyparentaldeath
– Adults(Sachs-Erickson,2017)– Childrenandadolescents(Nelson,2017)
• MechanismmaybeviaalteredHPAaxisandautonomicdysfunc:on(Elbers,2017)
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Set Realistic Goals • Pa:entavoidsdisability/reversesdisability• Fewer“bad”days• Selftreatmentstrategiesreduceneedformedicalinterven:on
• Increasedself-efficacy
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Mindy’s Goals • Goodshoulderalignmentwithoutshouldersubluxa:on:60%met• Cantalkunlimitedbyjawpain/locking:Met• ImprovedstabilityBhipstoallow1hourofpainfreewalkingwith
restbreaks:Metwithpain,nopainflarewith30minutesofwalking• IndependentwithprogressiveHEPwithself-reliefforpain:Metfor
basicprogramforshoulders,neck,ribs,hips,core• Improvedbiomechanicsandstabilityofribcageforfull,painfree
ven:la:on:Mostlymet,selftreatsribmalalignment• Improvedmobilityandreducedpaininheadandneck:Partlymet,
needscuestostabilizeneckduringarmuse
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Pain Self-Management • Techniquestodecreasepain:
– Painneuroscienceeduca:on(e.g.,“ExplainPain”)– Cogni:vebehavioraltherapy– Relaxa:on– Self-carewithheat,ice,TENS
• Techniquestodecreaseinjury– Jointprotec:onstrategies– Bracing/splin:ng,etc.
149 PT for Hypermobility Spectrum Disorders
Body Mechanics • Postureandergonomicsatschool/work/home• Sleepingposture,surface,support– Engagingmusclesbeforemoving– Assis:vedevices(e.g.,pens,tools,etc.)
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Biomechanical Differences • Shoulderse~ng
– Cueupinsteadofdown– Ballbackinsocket,notjustscapulae–cueupsidedown“G”
• Bicepslongheadsubluxa:on– ReduceERandabduc:onposi:ons
• Hipposteriorsea:ng– Nohipflexorusewithoutglutuseor– Alterposi:onofexercisetoassiststability/posteriorsea:ng
suchasinlongsi~ng
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Biomechanical Differences • Ankleprotec:on
– Bedsheetscansubluxtalusanteriorly– Si~ngonankles
• Patellofemoralinstability– TypicalissuesOsgoodSchla|er’s,trackingissues
• Elbow–ulnarnervesubluxa:onusuallyat90+degrees
• Wrist–carpalsubluxa:ons,rota:ons
152 PT for Hypermobility Spectrum Disorders
Biomechanical Differences • Craniocervicalinstability
– Odenjustlongholdintomobiliza:ondirec:onisenoughtonormalize
• SpineInstabili:es– Spondylolisthesis– Retrolisthesis
• Ribse~ng– ½inhalebeforeliding– Ifdepressedribs,inhalewithac:vity– Ifelevatedribs,exhalewithac:vity
153 PT for Hypermobility Spectrum Disorders
Exercise: Do No Harm! • Researchshowsthatmanypa:entswithhEDShavenega:vepastphysicaltherapyexperiencesdueto:
– Iatrogenicjointinjuries
– Unmetrehabilita:onneeds» (Bovet,2016)
154 PT for Hypermobility Spectrum Disorders
Exercise: Do No Harm! Avoidmechanicalpain
• Stabiliza:on&motorcontrolarecri:calThinkbeforemoving
• Tensilestrengthof:ssuesvarieswithac:vity,menses,inflammatorystate,ageetc.
• Slowprogressiontoallowhistologicalchanges
155 PT for Hypermobility Spectrum Disorders
Exercise: Do No Harm!
Cau:onwithoveruse:nerveentrapment,triggerpointsCau:onwithchangingforces
e.g.,exercisebandsincreaseresistance
Allow:ssuerecoverybetweenexercisebouts
2-hourrule:discomfortshouldreturntobaselinewithin2hours
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Exercise Progression • Rememberbasicprinciplesofexerciseprogression
157 PT for Hypermobility Spectrum Disorders
Proprioceptive Exercise • Propriocep:onsignificantlyworseinkneeswithBJHS(Sahin,2008)
– Propriocep:onexercisesimprovepainandfunc:on(Sahin,2008)• Propriocep:veexforkneesreducespainandimprovesqualityof
life(Ferrell,2004)– ClosedChainLEex–bridges,squats,sidelungetos:mulatejoint
receptors• Neckandspinepain-Propriocep:veexmaybelessrelevantthan
behavioralandeduca:onalRx,metaanalysis(McCaskey,2014)
• Backpainreduc:on,muscleenduranceandposturalstabilityimprovementswithlumbarspinalstabexercise(ToprakCelenay,2017)
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Exercise: Proprioception • Externalfocusexercises–putalaser
onitanddrawonthewall,thinkBAPS(SenMoCorSystem™)
• Biofeedback(e.g.,Stabilizer™)• Alterna:ngisometrics,dynamic
stabiliza:on• Ballexercises,balance&
propriocep:on• Emphasizegoodmotorcontrol
159 PT for Hypermobility Spectrum Disorders
Exercise Progression Easier
• Morepropriocep:vefeedback– Tac:le,visual,verbal– Externalfocus,e.g.lasers
• Moresupport– Exercisemachines– Againstwall
Harder
• Lessexternalpropriocep:vefeedback
• Lesssupport– Morechallenge,e.g.,unstable
surfaces– Freeweightsorbands
160 PT for Hypermobility Spectrum Disorders
Exercise Progressions Gravityassistedstabiliza:on,maximumtac:lefeedback
Addinggravitytostabilizerswithoutchallengingsubluxa:on
PT for Hypermobility Spectrum Disorders 161
Exercise Progressions Notowelunderthearm,set
upandback,controlresistanceReducingtac:le
feedback/useofwall
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Exercise Progression Easier
• Focusonjointstability– Isolatelegorarmwithout
spinemovement– Isolateshoulderstabilizers– Isolatecore
• Visualiza:on/mind-body– E.g.,jointcompression,
Qigong
Harder
• Focusonintegra:on– Coordinatecoreandlimb
movement– Addexternalchallenge– Complexmovements,e.g.,TaiChi
• Adddistrac:on/mul:tasking
163 PT for Hypermobility Spectrum Disorders
Exercise Progressions Speed/alterna:ngisometrics Againstgravityismoreadvanced
PT for Hypermobility Spectrum Disorders 164
Stabilization Exercises
• Shoulderse~ngwithfeedback
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Exercise Progression – Central Sensitization
Easier
• ChronicPain–unstablepla]orm,flowingmo:on,alternateagonist/antagonistorcontract/relaxandstretch
• Externalfocusexercise–laserpointerontarget/visualiza:on
Harder
• ChronicPain-longerholds,isometrics,longersets,stretchlaterinsession
• Focusonbodyposi:on
166 PT for Hypermobility Spectrum Disorders
Craniocervical Flexion vs Proprioception Ex
• Group1exercisingwithStabilizer/longuscolinods• Group2exercisingwithlaseronheadandeyeandheadmovementstotargets(externalfocus)
• BothGroupshadreduc:oninpainandneckdisabilityindex
• Propriocep:ongroup>CCflexionimprovedtolerancetotriggerpointpalpa:on
(Galllego-Izquierdo,2016)
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Exercise Progression Easier
• Midrange• Shortdura:on• Slow• Lowimpact• Symmetrical/bilateral • RPEstartat4/10
Harder
• FullRange• Longerdura:on• Fast• Addimpact• Unilateral/asymmetrical• RPE7.5/10ul:mategoal
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Exercise: Strengthening • Strengthenstabiliza:onmmbeforeglobalmm
– SpinestabilityexwithBJHSreducedpain,improvedendurance,improvedposturalstability(ToprakCelenay,2016)
• Possibilityofincreasedjoints:ffnesswithincreasedstrength– Cheerleadersincreasedshoulders:ffness,decreasedant.capsulelaxity(Laudner,2013)– Increasedpatellartendons:ffnessinpa:entswithcEDS(Moller,2014)– Mechanicalstraininhibitscollagenbreakdown(Flynn,2010)
• Needpropriocep:on&motorcontrol(Scheper,2016)• Beawareofstresseson‘incidental’joints,e.g.
– HandsgrippingweightsorTheraband– Wristextensioninquadruped– Spinestabiliza:onforextremityexercise
169 PT for Hypermobility Spectrum Disorders
Cardiovascular Exercise • Chronicpainleadstodecondi:oning• Exercise-inducedanalgesiacanreducepain• Startcardioaderini:alcorestabiliza:ontrainingorusemachinesthatprovidestability(e.g.,recumbentbike)
• Makeitfunsopa:entss:ckwithit170 PT for Hypermobility Spectrum Disorders
Exercise: Stretching • PeoplewithEDScanhave:ghtstructures• Isolatestretchtoproperstructure
– Keepjointsinproperalignmentwhenstretchingmuscles
• Stretchesmayneedtobegentle– Onlydo80%stretchandhold3-5secinpa:entswithperipheralsensi:za:on
– Thiscanminimizeflaresinsensi:zedpa:ents
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Bracing and Taping • Mul:direc:onalinstabilitycommon• Hips,Shoulders,SI,Knees,Ribs,
Fingers,Feet…• Benefitmaybefrompropriocep:ve
feedback• Givepa:entresourcestoselfmanage
painfulareasastheyarise• Teachpa:enttousemusclestobrace
fortheac:vity
172 PT for Hypermobility Spectrum Disorders
Picture of Mindy’s Modified Brace
PT for Hypermobility Spectrum Disorders 173
Manual Therapy • Relievemmspasm,TrP,&fascialadhesions• Decreasepain&autonomictone• Realignjointscarefully
– MWM,MET,nervemobs,gradesI-IV– Stabilizenearbystructures
• DONOHARM!– Donotover-mobilize
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Precautions and Red Flags • Spinalfluidincreasedpressure,leaks,syrinxesandheadaches–
– Mindyhaddocumentedleakfromnoseapprox.1cupofspinalfluidaderhusbandpushedonherupperneckwithmassagewhichalleviatedher“worstheadacheinherlife”
• Craniocervicalinstability–isdizzinessfromPOTSorCCI,stress,BPPV?• ChiariMalforma:on–maycauseincreasedspinalfluidpressure,ataxia
especiallyifmalallignedinuppercervical• Tetheredcord–avoidexcessivenervetension/flossingonly
– Saddleanesthesia,difficultywalking,bowel/bladderissues,tension• Precau:onsaswithapregnantpa:entforlaxity
PT for Hypermobility Spectrum Disorders 175
Mindy’s outcomes • 22%increaseinfunc:ononCareConnec:onsform• 4.5/7globalra:ngofchangeonscaleof-7to+7• Worstpainreducedfrom9/10to4-5/10,LBP0/10• Areastreated:Neck,jaw,ribs,shoulder,hips,LB• 21visitsover6months
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Summary of Adult Case • Needcomprehensiveevalua:on• Challengetoovercomegravity• Motorcontrolisessen:al• Subtlechangescanbeimportant• Startlow,goslow!
PT for Hypermobility Spectrum Disorders 177
Overall Summary • Hypermobilityspectrumdisordersarecommon• HSDinvolvesmanybodysystems,notjustjoints• Physicaltherapyiskeytomanagement• Programmustbecustomizedtothepa:ent• Lookforzebras!
178 PT for Hypermobility Spectrum Disorders
PT for Hypermobility Spectrum Disorders 179 PT for Hypermobility Spectrum Disorders 180
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